In patients with cortisone-reductase deficiency, the mutations in HSD11B1 or hexose-6-phosphate dehydrogenase (H6PD) that encodes an enzyme furnishing cofactors for the reaction might abrogate cortisol generation and consequently stimulate adrenal hyperandrogenism mediated by adrenocorticotropic hormone [25,26]. The gene discussed is POMC; the disease is hyperinsulinemic hypoglycemia, familial, 4.